РЕДКИЕ И СЛОЖНЫЕ ГЕМАТОЛОГИЧЕСКИЕ СИНДРОМЫ

Особенности диагностики и лечения тромботической тромбоцитопенической пурпуры, развившейся во время беременности: обзор литературы и собственное наблюдение

РЕДКИЕ ГЕМАТОЛОГИЧЕСКИЕ ЗАБОЛЕВАНИЯ И СИНДРОМЫ, РЕДКИЕ И СЛОЖНЫЕ ГЕМАТОЛОГИЧЕСКИЕ СИНДРОМЫ , , , , , , , ,

В.В. Войцеховский1, Л.Б. Филатов2, А.В. Пивник3, П.В. Авдонин4, Т.В. Есенина5, А.Г. Судаков5

1 Амурская государственная медицинская академия, ул. Горького, д. 95, Благовещенск, Амурская обл., Российская Федерация, 675000

2 Екатеринбургский консультативно-диагностический центр, Суворовский пер., д. 5, Екатеринбург, Свердловская обл., Российская Федерация, 620039

3 Московский клинический научно-практический центр им. Д.Д. Плетнева Департамента здравоохранения, ш. Энтузиастов, д. 86, Москва, Российская Федерация, 111123

4 Институт биологии развития им. Н.К. Кольцова, ул. Вавилова, д. 26, Москва, Российская Федерация, 119334

5 ГАУЗ АО «Амурская областная клиническая больница», ул. Воронкова, д. 26, Благовещенск, Амурская обл., Российская Федерация, 675028

Для переписки: В.В. Войцеховский, д-р мед наук, доцент, ул. Горького, д. 95, Благовещенск, Амурская обл., Российская Федерация, 675000; тел.: +7(4162)42-94-97; e-mail: voitsehovsckij@yandex.ru

Для цитирования: Войцеховский В.В., Филатов Л.Б., Пивник А.В., Авдонин П.В., Есенина Т.В., Судаков А.Г. Особенности диагностики и лечения тромботической тромбоцитопенической пурпуры, развившейся во время беременности: обзор литературы и собственное наблюдение. Клин. онкогематол. 2014; 7(4): 587–598.

РЕФЕРАТ

В работе отражены современные представления об этиологии, патогенезе, клинических особенностях и диагностике тромботической тромбоцитопенической пурпуры (ТТП или болезнь Мошковица). Обобщены сведения о редкой патологии — наследственной ТТП (синдром Апшо—Шульмана), ее манифестации в период беременности. Освещены различные аспекты дифференциальной диагностики микроангиопатических гемолитических анемий, сложности дифференциальной диагностики тромботических микроангиопатий (ТТП, гемолитико-уремический синдром, HELLP-синдром) во время беременности. Рассмотрены особенности клинического течения и дифференциальной диагностики приобретенной и наследственной ТТП у беременных. Подробно изложены вопросы лечения ТТП во время беременности.

Ключевые слова: тромботическая тромбоцитопеническая пурпура, наследственная тромботическая тромбоцитопеническая пурпура, синдром Апшо—Шульмана, тромботическая микроангиопатия, микроангиопатическая гемолитическая анемия, шистоциты, тромбоцитопения, ADAMTS-13, HELLP-синдром, беременность.

Принято в печать: 3 октября 2014 г.

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Аутоиммунный лимфопролиферативный синдром в гематологической клинике: обзор литературы и собственное наблюдение

РЕДКИЕ И СЛОЖНЫЕ ГЕМАТОЛОГИЧЕСКИЕ СИНДРОМЫ , , , , , , ,

И.Б. Ковынев1, Т.И. Поспелова1, А.В. Мишенин2, И.Н. Нечунаева2, Р.В. Тарновский1

1 ГБОУ ВПО «Новосибирский государственный медицинский университет», Новосибирск, Российская Федерация

2 МБУЗ «Городская клиническая больница № 2», Новосибирск, Российская Федерация

РЕФЕРАТ

В статье представлены обзор литературы и описание редкого наблюдения аутоиммунного лимфопролиферативного синдрома (ALPS — autoimmune lymphoploliferative syndrome). Заболевание связано с блоком апоптоза иммунных клеток, вызванного мутацией генов Fas-протеинов. Клинически процесс характеризуется прогрессирующим генерализованным лимфопролиферативным синдромом при картине реактивной гиперплазии в биоптате лимфоузлов и иммунными 1–3 ростковыми цитопениями. Обсуждаются вопросы трансформации в лимфому или системную красную волчанку и современной тактики лечения.

Ключевые слова: аутоиммунный лимфопролиферативный синдром, цитопении, аутоиммунная гемолитическая анемия, аутоиммунная нейтропения, лимфаденопатия, лимфопролиферативные заболевания, иммунная тромбоцитопеническая пурпура, лимфома.

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